Relapse of Wegener's granulomatosis. Concerning a case after 20 years of remission

Immunosuppressive drugs have transformed the prognosis of systemic Wegener' s granulomatosis. Nowadays, the main residual problem is illness relapses, for which management is largely undefined. We describe the case of a patient, aged 47 in 1977. The diagnosis of Wegene r's granulomatosis was made when faced with polyarthralgias, cutaneous vasc ulitis, rhinitis, dyspnea, hemoptysis and global decline of her physical co ndition. The treatment associated high-dose corticotherapy and intramuscula r cyclophosphamide for 1 year. This treatment led to a complete remission. Twenty years later, the patient was hospitalized for reoccurrence of rhinit is, dyspnea and right knee effusion associated with biological inflammatory syndrome, renal insufficiency and antibodies against polymorphonuclear neu trophil cytoplasm, type c-ANCA. Chest CT-scan disclosed parenchymal infiltr ates. Wegener relapse was diagnosed and the combination of three methylpred nisolone perfusions followed by oral prednisone (1 mg/kg/d) and a monthly b olus of cyclophosphamide led to a new remission. Nevertheless, 4 months aft er beginning the treatment the patient died from an infectious complication (Pneumocystis carinii and aspergillosis). Relapses of Wegener's granulomatosis are frequent and difficult to predict. Moreover, some Gases occur very early. The remarkable efficiency of cyclop hosphamide to induce remission is however shaded by the high rate of relaps e. Other drugs are studied to identify more efficient therapy, able to both induce remission and prevent relapses, but reliable data are still missing to determine the best therapeutic regimen. Joint Bone Spine 2001 ; 68 : 26 2-6. (C) 2001 Editions scientifiques et medicales Elsevier SAS.